HIRSCHSPRUNG's disease with aganglionosis of the lower small intestine as well as the entire colon presents a particular challenge to the surgeon. Eight years ago we encountered an infant with aganglionosis of the entire colon and approximately half the small bowel. The surgical procedure which we ultimately employed to cope with this desperate situation proved to be successful, and the child is now leading a normal life. Since then, the operation has been performed on four additional infants with aganglionosis of the entire colon and terminal small bowel with uniform success.
About 5% of infants with Hirschsprung's disease will have the entire colon aganglionic. Extensive aganglionosis of the small bowel in addition is less frequent. Without surgery, most of these infants will die during the first few weeks of life although occasionally, one may live to be several years of age.1,2 A recent collective review of 29 cases